Prevalence of portal vein obstruction in pediatric patients after liver transplantation; a multicenter, observational, prevalence study
Bader Alfares1,2, Reinoud P.H. Bokkers1, Ruben H.J. de Kleine3, Hubert P.J. van der Doef4.
1Department of Radiology, University Medical Center Groningen, Groningen , Netherlands; 2Department of Radiology, King Faisal Specialist Hospital and Research Cente, Riyadh, Saudi Arabia; 3Department of Surgery, University Medical Center Groningen, Groningen, Netherlands; 4Department of Pediatrics, University Medical Center Groningen, Groningen, Netherlands
The PORTAL Registry.
Introduction: Portal vein obstruction (PVO) including portal vein thrombosis (PVT) and portal vein anastomotic stenosis (PVAS) are common in patients after pediatric liver transplantation (pLT). However, prevalence data within the high risk groups (biliary atresia (BA), living-donor pLT, and age pLT <1 year) are lacking. The aim of the study was to assess the overall prevalence of PVO after pediatric liver transplantation and occurrence within various predefined high risk group.
Methods: The Portal vein Obstruction Revascularisation Therapy After Liver transplantation (PORTAL) registry is a multicenter, retrospective, observational registry of PVO after pLT among 15 pediatric liver transplantation centers. Herein, the prevalence of PVO was determined using the number of patients with PVO within the whole population of pLT and in specific subgroups who were transplanted between 1 January 2001 and 1 January 2020. Moreover, subgroup analysis was determined in predefined high risk groups. Differences were calculated using the chi-square test.
Results: In total, 4961 transplanted patients were included across 15 centers (response rate=74%); Europe (67%), North America (13%), and South America (20%). Of the total transplanted patients, 40% had BA (n=1973), 24% had a living donor liver transplantation (n=1193), and 26% had an age at transplantation of <1 year (n= 1306) with mean age pLT of 2.2 years (SD 2.9). Overall, PVO prevalence was 6.4% (320/4961), of which 164 patients with PVAS (3.3%), 128 with PVT (2.5%), and 28 with both (0.5%). Prevalence of PVO within high risk groups was: 11% in biliary atresia, 13.7% in living-donor, 11.8% in transplanted age of <1 year. Among the highest risk group (patients with BA transplanted <1 year), PVO was significantly more prevalent in patients transplanted with a living liver donor (19.6%; 84/428) in comparison with patients transplanted with deceased liver donor (9.2%; 48/519; p<0.001).
Conclusion: Within the PORTAL registry, the overall prevalence of PVO is 6.4%. Among BA patients transplanted below 1 year of age, patients transplanted with living liver donor had the highest prevalence of PVO of nearly 20%. Future studies on PVO, specifically prevention and management, should focus on these high risk patients.
Barbara E. Wildhaber. Stéphanie Franchi-Abella. Thomas Casswall. Paolo Marra. Guillermo Cervio. Martín de Santibañes. Jai V. Patel. Catalina Jaramillo. Piotr Kaliciński. Jesús Q. Bernabeu. Mureo Kasahara. Vidyadhar Mali. Helen Evans. Steffen Hartleif. Cristina T. Ferreira. Amit A. Shah. Ryan T. Fischer. Rajeev Khanna. Khalid Sharif. Georg F. Vogel. Winita Hardikar.
[1] Piardi T, Lhuaire M, Bruno O, et al. Vascular complications following liver transplantation: A literature review of advances in 2015. World J Hepatol. 2016;8(1):36-57.
[2] Stevens JP, Xiang Y, Leong T, et al. Portal vein complications and outcomes following paediatric liver transplantation: data from the Society of Paediatric Liver Transplantation. Liver Transpl. 2022;00:1–11.
[3] Yabuta M, Shibata T, Shibata T, et al. Long-term outcome of percutaneous transhepatic balloon angioplasty for portal vein stenosis after pediatric living donor liver transplantation: a single institute's experience. J Vasc Interv Radiol. 2014;25(9):1406-12.
[4] Shibasaki S, Taniguchi M, Shimamura T, et al. Risk factors for portal vein complications in pediatric living donor liver transplantation. Clin Transplant. 2010;24(4):550-556.
[5] Alvarez F. Portal vein complications after pediatric liver transplantation. Curr Gastroenterol Rep. 2012;14:270–74.
[6] Ueda M, Egawa H, Ogawa K, et al. Portal vein complications in the long-term course after pediatric living donor liver transplantation. Transplant Proc. 2005;37:1138–40.
[7] Karakayali H, Sevmis S, Boyvat F, et al. Diagnosis and treatment of late-onset portal vein stenosis after pediatric living-donor liver transplantation. Transplant Proc. 2011;43:601–4.
[8] Naik KB, Hawkins CM, Gill AE, et al. Clinical efficacy of percutaneous Transhepatic portal vein angioplasty for late-onset portal vein stenosis in pediatric liver transplant patients. Transplantation. 2018;102:e282–7.
